Spontaneous Remission in Paroxysmal Nocturnal Hemoglobinuria-Return to Health or Transition Into Malignancy?

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Korkama , E-S , Armstrong , A-E , Jarva , H & Meri , S 2018 , ' Spontaneous Remission in Paroxysmal Nocturnal Hemoglobinuria-Return to Health or Transition Into Malignancy? ' , Frontiers in Immunology , vol. 9 , 1749 . https://doi.org/10.3389/fimmu.2018.01749

Title: Spontaneous Remission in Paroxysmal Nocturnal Hemoglobinuria-Return to Health or Transition Into Malignancy?
Author: Korkama, Eva-Stina; Armstrong, Anna-Elina; Jarva, Hanna; Meri, Seppo
Contributor: University of Helsinki, Research Programs Unit
University of Helsinki, Hematologian yksikkö
University of Helsinki, Doctoral Programme in Biomedicine
University of Helsinki, HUSLAB
Date: 2018-08-02
Language: eng
Number of pages: 5
Belongs to series: Frontiers in Immunology
ISSN: 1664-3224
URI: http://hdl.handle.net/10138/238980
Abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired syndrome characterized by intravascular hemolysis, thrombosis, and bone marrow failure. The disease is caused by a mutation in the PIG-A gene that leads to the lack of glycosylphosphatidylinositol-anchored complement regulatory molecules CD55 and CD59 on affected blood cell surfaces. In previous studies, spontaneous clinical remissions have been described. The disease manifestations are very heterogeneous, and we wanted to examine if true remissions and disappearance of the clone occur. In a follow-up of a nation-wide cohort of 106 Finnish patients with a PNH clone, we found six cases, where the clone disappeared or was clearly diminished. Two of the patients subsequently developed leukemia, while the other four are healthy and in clinical remission. According to our data, spontaneous remissions are not as frequent as described earlier. Since the disappearance of the PNH cell clone may indicate either a favorable or a poor outcome-remission or malignancy-careful clinical monitoring in PNH is mandatory. Nevertheless, true remissions occur, and further studies are needed to understand the immunological background of this phenomenon and to obtain a better understanding of the natural history of the disease.
Subject: paroxysmal nocturnal hemoglobinuria
aplastic anemia
spontaneous remission
leukemia
MEMBRANE-PROTEIN
DEFICIENCY
ERYTHROCYTES
3111 Biomedicine
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